Abstract
The authors summarized the results of ten years care of adult patients with cystic fibrosis (CF). They treated a total of 45 patients incl. 26 men and 19 women.
Nineteen patients (42%) died. The most frequent cause of admission to hospital was respiratory infection (84%), planned antibiotic treatment (6.5%), pneumothorax (6%) and haemoptysis (4%).
In all patients impaired ventilation of the obstructive type was found. The mean annual decline of FEV1 was 0.173 +/- 0.194 l, VC 0.303 +/- 0.323 l.
The authors did not find a significant difference between live and dead patients. The mean annual increase of PaCO2 was 0.5 +/- 0.87 kPa, the mean annual decrease of PaO2 was 0.6 +/- 0.96 kPa.
The difference of values in dead and live patients was significant. CF still remains an incurable disease.
It is, however, possible by comprehensive treatment to prolong the patients life and to improve its quality.